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Malignant melanotic nerve sheath tumor (previously known as melanotic schwannoma) is a rare aggressive peripheral nerve sheath tumor that typically develops in conjunction with spinal or visceral autonomic nerves, consisting uniformly of Schwann cells displaying melanocytic differentiation.
New and more precisely defined entities include malignant melanotic nerve sheath tumor (formerly known as melanotic schwannoma) and hybrid nerve sheath tumors. [4] [5] The majority of peripheral nerve tumors are benign tumors of the nerve sheath (usually schwannomas); on rare occasions, they are metastatic tumors or originate from the nerve cells.
Malignant schwannoma, [1] Neurofibrosarcoma, [1] and Neurosarcoma [1] Micrograph of malignant peripheral nerve sheath tumour with the typical herringbone pattern . H&E stain .
Spinal nerve sheath tumors generally arise as single lesions. [1] Presence of multiple lesions is associated with genetic conditions including neurofibromatosis type 1, neurofibromatosis type 2, and Schwannomatosis. [1] [3] [4] Most spinal schwannomas are intradural-extramedullary, growing inside the thecal sac, but outside the spinal cord ...
1.6.3.1 Spinal ependymoma, MYCN-amplified ... 5.1 Schwannoma ... 5.4 Hybrid nerve sheath tumour 5.5 Malignant melanotic nerve sheath tumour
Schwannomatosis is an extremely rare genetic disorder closely related to the more-common disorder neurofibromatosis (NF). Originally described in Japanese patients, [1] it consists of multiple cutaneous schwannomas, central nervous system tumors, and other neurological complications, excluding hallmark signs of NF.
Most symptoms from spinal tumors occur due to compression of the spinal cord as it plays a primary role in motor and sensory function. [7] The spinal cord is surrounded by three layers known as the spinal meninges. [7] These are the dura mater, arachnoid mater, and pia mater. Spinal cord tumors are classified based on their location within the ...
Micrograph of a psammomatous melanotic schwannoma with a psammoma body, as may be seen in Carney complex. H&E stain. Psammoma bodies may be seen in: Endosalpingiosis [10] Psammomatous melanotic schwannoma; Melanocytic nevus [11]